antibody

Goat anti-human von Willebrand Factor, Whole IgG from antiserum

MBS512174

2.5 mg

245 USD

polyclonal

goat

nonconjugated

Antibody

Goat anti-human von Willebrand Factor, Whole IgG from antiserum

von Willebrand Factor

von Willebrand Factor, human

von Willebrand factor; von Willebrand factor; von Willebrand factor

VWF; VWF; F8VWF; F8VWF

VWF_CANFA

Polyclonal

Goat

This antibody is specific for vWF as demonstrated by immunoelectrophoresis and ELISA.

Purified IgG. Vial containing ml of whole IgG representing approximately 1 ml of antiserum. Total protein is 2.5 mg.

Whole IgG, clear liquid.

IgG concentration is mg/ml, determined by absorbance using an extinction coefficient (E1%280) of 13.4.

Store between -10 and -20 degree C. Product will become viscous but will not freeze. Avoid storage in frost-free freezers. Keep vial tightly capped. Allow product to warm to room temperature and gently mix before use.

Immunogen: Human vWF purified from plasma.

Buffer: 10 mM HEPES, pH 7.4, 150 mM NaCl, 50% (v/v) glycerol.

von Willebrand Factor (vWF, also previously referred to as Factor VIII related antigen) is a large adhesive protein produced in endothelial cells and megakaryocytes. There are two critical functions of vWF, the first being its involvement in the process of platelet adhesion and aggregation through interaction with platelet receptor glycoprotein Ib, the second being the binding and stabilization of Factor VIII (antihemophilic factor) for secretion and transport in plasma. The vWF precursor protein is synthesized with a 95,000 dalton propeptide (also known as vWF antigen-II), believed to be involved in the intracellular multimerization of the vWF subunits. The mature vWF multimers are then packed into storage organelles within the cell (Weibel-Palade bodies) after which the propeptide is cleaved and released. vWF circulates as multimers of disulphide linked 220,000 dalton subunits and the molecular weight of these multimers ranges from 0.5- 20 million daltons. The plasma concentration of vWF is typically 10 ug/ml, but increased levels are often observed in pregnancy and other conditions of physiological stress. von Willebrand's disease (vWD) is perhaps the most common inherited bleeding disorder in humans and is the result of either quantitative deficiencies of vWF (vWD Types I & III), or one of a number of qualitative disorders of vWF structure and function (vWD Type II).

1. Montgomery RR, Coller BS, in Hemostasis and Thrombosis, 3rd Edition, eds. RW Colman, J Hirsh, VJ Marder and EW Salzman, pp. 134- 168, J.B. Lippincott Co., Philadelphia PA,.USA, 1994. 2. Sadler JE; von Willebrand Factor (Minireview); JBC 266:34, pp 22777- 22780, 1991.

50950127

NP_001002932.1

NM_001002932.1

Q28295

309533 Da

Blood Clotting Cascade Pathway 198608!!Complement And Coagulation Cascades Pathway 84123!!Complement And Coagulation Cascades Pathway 484!!ECM-receptor Interaction Pathway 84118!!ECM-receptor Interaction Pathway 479!!Focal Adhesion Pathway 84117!!Focal Adhesion Pathway 478!!Formation Of Platelet Plug Pathway 363620!!GRB2:SOS Provides Linkage To MAPK Signaling For Intergrins Pathway 363617!!Hemostasis Pathway 363619

Function: Important in the maintenance of hemostasis, it promotes adhesion of platelets to the sites of vascular injury by forming a molecular bridge between sub-endothelial collagen matrix and platelet-surface receptor complex, glycoprotein Ibalpha/IX/V. Also acts as a chaperone for coagulation factor VIII, delivering it to the site of injury, stabilizing its heterodimeric structure and protecting it from premature clearance from plasma. Subunit structure: Multimeric. Interacts with F8. Ref.2. Subcellular location: Secreted. Secreted extracellular space extracellular matrix. Note: Localized to storage granules. Ref.2. Tissue specificity: Plasma. Domain: The propeptide is required for multimerization of vWF and for its targeting to storage granules. Post-translational modification: All cysteine residues are involved in intrachain or interchain disulfide bonds. By similarity.N- and O-glycosylated. Involvement in disease: Note=Defects in VWF are the cause of von Willebrand disease (VWD) in the Scottish Terrier. VWD is characterized by frequent bleeding. Type I VWD is associated with a deficiency of VWF; type II by normal to decreased plasma level of VWF; type III by a virtual absence of VWF. Ref.4. Sequence similarities: Contains 1 CTCK (C-terminal cystine knot-like) domain.Contains 4 TIL (trypsin inhibitory-like) domains.Contains 3 VWFA domains.Contains 3 VWFC domains.Contains 4 VWFD domains.

2.5 mg

245 USD